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Glycosaminoglycan synthesis in skin fibroblasts from patients with osteogenesis imperfecta
Authors:R Kapoor  S Bourier  P Prehm
Institution:Department of Bacteriology and Epizootology, College of Veterinary Medicine, University of Agricultural Sciences, Biomedical Center, Box 583, S-751 23 Uppsala, Sweden;Department of Chemistry I, Karolinska Institutet, S-104 01 Stockholm, Sweden
Abstract:Glycosaminoglycans were analysed from skin fibroblasts with osteogenesis imperfecta (OI) IIA and IIB. The content of sulphated glycosaminoglycans was greatly increased over age-matched controls and to a lesser extent with respect to older age control. Dermatan sulphate in comparison with older control was unaltered in the cells of OI IIA and IIB. The concentration of heparan sulphate was higher in the cells than in the medium, whereas hyaluronic acid, chondroitin sulphate and dermatan sulphate content was higher in the medium. The level of hyaluronic acid was greatly elevated in the medium of OI IIB with respect to both controls.
Keywords:Heat-sta-ble enterotoxin  Amino acid sequence  Compensated amino acid exchange
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